Protein C Infusion Therapy in an Adult Case of Severe Congenital Protein C Deficiency
Bibliographic record
Abstract
Abstract Background: Severe congenital protein C deficiency (SCPCD) is a rare inherited disorder characterized by recurrent thrombosis, disseminated intravascular coagulation, and purpura fulminans. Due to limitations in traditional therapeutic options (anticoagulation, FFP, PCC), plasma-derived protein C concentrates have been used for both prophylactic and therapeutic indications. However, there are few reports of protein C infusion therapy in adults, with limited understanding of pharmacokinetics and no dosing guidelines available. We report the use of protein C concentrate in an individualized dose-finding study, in an adult patient in preparation for renal transplantation. Case: A 35 year old female (68 kg) with SCPCD (baseline PC activity Results: The patient was admitted to hospital for this dose-finding study. She was administered Ceprotin® (human plasma-derived, virally-inactivated protein C concentrate) with an initial intravenous bolus (59 IU/kg) followed by an infusion (5 IU/kg/hr) to maintain a target PC level of 0.65-1.30 U/mL (see Table). Protein C activity was measured every 6 hours, and target levels were achieved over the 30-hour duration of the trial. The patient experienced no adverse events during the infusion trial. Conclusion: In this case report, target protein C levels were maintained using bolus and continuous infusion rates of protein C concentrate that were approximately half of those recommended by the manufacturer. This personalized trial, while resource-intensive, provided a feasible and safe method of preparing for a high-risk surgical procedure that could be adapted to other similar clinical situations. Download : Download high-res image (89KB) Download : Download full-size image Table . Disclosures Crowther: Leo Pharma: Research Funding; Shinogi: Consultancy; Boehringer Ingelheim: Speakers Bureau; Bayer: Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Alexion: Speakers Bureau; Pfizer: Honoraria; Portola: Consultancy.
Fetched live from OpenAlex and de-inverted. Abstracts are not stored in this database: the inverted indexes are 8.6 GB of the frame’s 9.3 GB of text, and the host has 13 GB free.
How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.002 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.003 | 0.003 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".